Cardiomyopathies

Question 1

Mitral doppler findings RCM

Answer 1

Fast E wave, stops abruptly

Almost no A wave

Question 2

PV doppler RCM

Answer 2

Very little systolic flow

Rapid inflow in early diastole, stops abruptly

Question 3

Treatment of RCM

Answer 3

Cautious diuresis
Cautious BB
May need transplant

Question 4

Amyloid features

Answer 4

Very thick walls
Speckled appearance
EKG normal or low voltage

Question 5

Hypereosinophilia features

Answer 5

Fuzzy around myocardium, thrombus-like deposition in apices
If mobile elements -> anticoagulant
Hydroxyurea, steroids

Question 6

ARVD features

Answer 6

Fatty replacement of RV free wall -> R heart failure
Arrhythmias
Repolarization abnormality, conduction delay
30% with FH
Often need ICD, no competitive athletics

Question 7

LV non-compaction features

Answer 7

Non-compacted myocardium
Crypts and recesses
Increased risk for LV dilation, CHF, possible SCD

Question 8

LV non-compaction and normal systolic function

Answer 8

treat as stage B HF

Use HCM / DCM criteria for SCD risk

Question 9

LV non-compaction and reduced systolic function

Answer 9

treat as stage B or C HF
A/C if EF < 35%
Use HCM / DCM criteria for SCD risk

Question 10

Ddx for thick walls

Answer 10

LVH
HCM
Renal failure
Amyloid
Glycogen storage disease
Anderson-fabry's disease
Freiderich's ataxia

Question 11

Athletic heart

Answer 11

LVH
LVEDD > 55 mm
Responds to de-training
Super-normal exercise capacity
No MRI scar tissue or perfusion defects

Question 12

EKG in apical HCM

Answer 12

Deep narrow T waves

Question 13

HCM echo screening in children

Answer 13

No later than onset of puberty or at any consideration of competitive athletics
Every 12-18 months

Question 14

HCM echo screening in adults

Answer 14

Every 5 years

Stop at 60 if normal

Question 15

Genetic testing for screening in HCM

Answer 15

If HCM patient has known mutation, genetic testing preferred for screening
If no known mutation, imaging necessary

Question 16

Pathophysiology of HCM

Answer 16

Diastolic dysfunction in 100% (increased LAP, dec CO)

Obstruction, SAM / MR in 70% (high LAP, subendocardial ischemia, MR, decreased CO)

Question 17

Significant gradient at rest in HCM

Answer 17

> 30

Question 18

Gradient to produce class III-IV symptoms in HCM

Answer 18

> 40-50

Question 19

HCM LVOT gradient worsens with

Answer 19

More vigorous contraction
Decreased resistance
Decreased volume

Question 20

Avoid in HCM

Answer 20

Positive inotropes
Pure vasodilators
High-dose diuretics

Question 21

Meds in HCM

Answer 21

Decrease contractility
Beta blockers
Verapamil, diltiazem
Disopyramide as adjunct

Question 22

Indications for myectomy / alcohol septal ablation in HCM

Answer 22

LVOT obstruction

Symptoms and impaired QoL despite meds

Question 23

Risk factors for SCD in HCM

Answer 23

NSVT
Massive LVH
FH 1st deg relative < 45
Unexplained syncope last 6 months
Abnormal BP response to exercise

Question 24

ICD indications for HCM class I

Answer 24

Prior cardiac arrest or sustained VT

Question 25

ICD indications for HCM class IIa

Answer 25

FH-SCD, LV thickness > 30mm, recent unexplained syncope | NSVT or abnormal BP response to exercise + risk modifiers

Question 26

Risk modifiers for HCM and ICD

Answer 26

``` Young Wall thickness close to 30 Remote FH SCD LVOT obstruction without planned intervention Delayed enhancement Septal ablation Known specific HCM mutations Syncope < 5 years ago ```

Question 27

Sarcoid cMRI

Answer 27

Midmyocardial and epicardial LGE, particularly in basal septum and basal lateral wall

Question 28

Fabry's disease

Answer 28

Preserved EF, chest pain, normal coronaries, LVH, renal dysfunction, FH (X-linked)

Question 29

CYP inhibitors which increase taco or calcineurin inhibitor levels

Answer 29

Azoles Diltiazem Verapamil

Question 30

pHTN 6MWT target for success

Answer 30

>380 m

Question 31

HF predictors of poor hospital prognosis

Answer 31

low SBP, elevated BUN, elevated Cr

Question 32

Dose for anthracycline toxicity

Answer 32

3-5% with 400 mg/m 7-26% at 550 mg/m 18-48% at 700 mg/m

Question 33

Risk factors for antracycline toxicity

Answer 33

1) intravenous bolus administration, 2) higher single doses, 3) history of prior mediastinal irradiation, 4) use of other concomitant agents known to have cardiotoxic effects, such as cyclophosphamide, trastuzumab, and paclitaxel, 5) female sex, 6) underlying cardiovascular disease, 7) extremes of patient age (both very young and old age), and 8) increased length of time since anthracycline completion.

Question 34

Diagnosis of familial cardiomyopathy

Answer 34

3 generations involved, genetic testing not necessary

Question 35

Screening for familial cardiomyopathy

Answer 35

1st degree relatives | Echo every 3-5 years

Question 36

Hereditary hemochromatosis

Answer 36

``` Iron overload (ferritin > 300, transferrin saturation >55%), T2 < 20 ms on CMR, Evidence of heart disease (reduced EF,r restrictive filling, pulmonary hypertension) ```

Question 37

IV iron in HF

Answer 37

Class II or III HF, ferritin < 100 or ferritin 100-299 and iron sat <20%

Question 38

ARVC EKG

Answer 38

TWI in anterior precordial leads, high-frequency, low amplitude deflection at end of QRS complex (epsilon waves)

Question 39

ARVC echo

Answer 39

RV dilatation, RV dysfunction

Question 40

ARVC mutation

Answer 40

AD | plakoglobin

Question 41

Killip 1

Answer 41

No signs of hF

Question 42

Killip 2

Answer 42

Rales (crackles) in the lungs, an S3, and elevated jugular venous pressure

Question 43

Killip 3

Answer 43

Acute pulmonary edema

Question 44

Killip 4

Answer 44

Cardiogenic shock or hypotension (systolic blood pressure <90 mm Hg) and evidence of peripheral vasoconstriction.

Question 45

Chemotherapy induced cardiomyopathy

Answer 45

decrease in cardiac LVEF that was either global or more severe in the septum HF symptoms Decline in LVEF of ≤5-55% with accompanying signs or symptoms of CHF or a decline in LVEF of ≤10-55% without accompanying signs or symptoms. 

Question 46

Cardiotoxic agents to monitor

Answer 46

anthracyclines, trastuzumab, sunitinib

Question 47

Tyrosine kinase inhibitors complications

Answer 47

Thrombotic events

Question 48

Vent to increase O2

Answer 48

FiO2, PEEP

Question 49

Vent to increase ventilation

Answer 49

RR, tidal volume